Al-Bderat Jwaher T, Mardinie Reham I, Salaita Ghazi M, Al-Bderat Amer T, Farrah Mahdi K
Department of Pediatric Nephrology, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan.
Department of Nephrology, King Hussein Medical Center, Amman, Jordan.
Saudi J Kidney Dis Transpl. 2017 Sep-Oct;28(5):1064-1068. doi: 10.4103/1319-2442.215138.
Nephrocalcinosis (NC) is defined as deposition of calcium crystals in the renal parenchyma and tubules. This is a retrospective review of all the data of 63 children presented to Pediatric Nephrology Clinic at King Hussein Medical Center (KHMC) over a 15-year period with bilateral NC. We determine their causes, clinical presentation and evaluate their growth and renal function during their follow-up. Thirty-five (55.5%) cases were males and 28 (44.5%) were females. The median (range) age at presentation was four (2-192) months. The most common leading cause to NC was hereditary tubulopathy in 48% followed by hyperoxaluria in 35%. The cause of NC remained unknown in 3% and Vitamin D excess accounts for 5% of the cases. The most presenting symptom was a failure to thrive (68%) and the second most common symptom was abdominal pain and recurrent urinary tract infection was found in 40%, polyuria and polydipsia were found in 32% of cases, and 16% of cases were diagnosed incidentally. At a median follow-up of 38 (14-200) months, estimated glomerular filtration rate had decreased from 84.0 (42-110) mL/min per 1.73 m2 body surface area to 68.2 (10-110) mL/min/1.73 m2 body surface (P = 0.001). This study illustrated the need for a national registry of rare renal diseases to help understand the causes of these conditions in our populations and provide support to affected patients and their families.
肾钙质沉着症(NC)的定义为钙晶体在肾实质和肾小管中的沉积。这是一项对15年间在侯赛因国王医疗中心(KHMC)儿科肾脏病诊所就诊的63例双侧NC患儿的所有数据进行的回顾性研究。我们确定了其病因、临床表现,并在随访期间评估了他们的生长发育和肾功能。35例(55.5%)为男性,28例(44.5%)为女性。就诊时的中位(范围)年龄为4(2 - 192)个月。导致NC最常见的主要原因是遗传性肾小管病,占48%,其次是高草酸尿症,占35%。3%的NC病因不明,5%的病例是维生素D过量所致。最常见的症状是生长发育迟缓(68%),第二常见症状是腹痛,40%的病例有反复尿路感染,32%的病例有多尿和烦渴,16%的病例是偶然诊断出来的。在中位随访38(14 - 200)个月时,估计肾小球滤过率从每1.73平方米体表面积84.0(42 - 110)毫升/分钟降至68.2(10 - 110)毫升/分钟/1.73平方米体表面积(P = 0.001)。这项研究表明需要建立一个罕见肾病国家登记处,以帮助了解我们人群中这些疾病的病因,并为受影响的患者及其家庭提供支持。